Abstract
The variation in standardized, well-defined parameters in Hirschsprung's disease (HSCR)
research hinders overarching comparisons and complicates evaluations of care quality
across healthcare settings. This review addresses the significant variability observed
in these parameters as reported in recent publications. The goal is to compile a list
of commonly described baseline characteristics, process and outcome measures, and
to investigate disparities in their utilization and definitions. A systematic review
of literature on the primary care process for HSCR was performed according to PRISMA
guidelines. Relevant literature published between 2015 and 2021 was obtained by combining
the search term “Hirschsprung's disease” with “treatment outcome,” “complications,”
“mortality,” “morbidity,” and “survival” in Medline, Embase, and the Cochrane Library.
We extracted study characteristics, reported process and outcome parameters, and patient
and disease characteristics. We extracted 1,026 parameters from 200 publications and
categorized these into patient characteristics (n = 226), treatment and care process characteristics (n = 199), and outcomes (n = 601). A total of 116 parameters were reported in more than 5% of publications.
The most frequently reported characteristics were sex (88%), age at the time of surgery
(66%), postoperative Hirschsprung-associated enterocolitis (64%), type of repair (57%),
fecal incontinence (54%), and extent of aganglionosis (51%). This review underscores
the pronounced variation in reported parameters within HSCR studies, highlighting
the necessity for consistent, well-defined measures and reporting systems to foster
improved data interpretability. Moreover, it advocates for the use of these findings
in the development of a Core Indicator Set, complementing the recently developed Core
Outcome Set. This will facilitate quality assessments across pediatric surgical centers
throughout Europe.
Keywords
Hirschsprung's disease - outcome - characteristics
